[A case of ganglioglioma extended to the lateral ventricle and associated with neurofibromatosis type 1].

We encountered a rare case of intraventricular ganglioglioma associated with neurofibromatosis type 1. A 42-year-old woman presented with a feeling of heaviness of the head and dizziness. She was diagnosed with neurofibromatosis type 1 because she had multiple subcutaneous neurofibromas and café au lait spots. On admission, she deteriorated slightly(Japan Coma Scale 1)and suffered from cognitive dysfunction and right hemiparesis. A computed tomography(CT)scan showed that she had an obstructed hydrocephalus with a long and circular mass lesion, 2cm in diameter, in the anterior horn of the left lateral ventricle. The mass showed low signal intensity(SI)on the T1-weighted image(WI), heterogeneous high SI on the T2-WI, and dense enhancement on a Gd-DTPA contrast MRI, extending from the head of the left caudate nucleus to the lateral ventricle. The patient underwent an urgent operation via an anterior transcallosal approach because of an obstructed hydrocephalus. The tumor was removed in its entirety, including its origin at the caudate head. The pathological diagnosis was a ganglioglioma grade 1 according with the classification of the World Health Organization. Here we describe this case and discuss the rare association between gangliogliomas and neurofibromatosis type 1.

A case of choroid plexus papilloma with stromal sclerosis and indistinct papillary structures.

We present a case of choroid plexus papilloma with unusual histopathological findings. A 41-year-old Japanese man presented with dizziness. Brain magnetic resonance imaging scan showed a distinct mass in the fourth ventricle. Histopathological examination showed proliferation of cuboidal cells forming irregular trabeculae, small nests, and pseudoglandular and indistinct papillary structures, accompanied by a variable degree of stromal sclerosis. The tissue sections also showed rare mitotic activity, no hypercellularity, cellular pleomorphism, or necrosis. The immunohistochemical findings were tumor cells positive for cytokeratin 7, vimentin, S-100 protein, and transthyretin but negative for cytokeratin 20, BerEP4 and carcinoembryonic antigen. These findings were consistent with choroid plexus papilloma. The combination of these immunohistochemical markers was helpful in establishing the diagnosis, although the morphological finding of stromal sclerosis is rare for choroid plexus papilloma.

Sonodynamic therapy with 5-aminolevulinic acid and focused ultrasound for deep-seated intracranial glioma in rat.

BACKGROUND: 5-Aminolevulinic acid (5-ALA) has already been applied clinically as a photosensitizer. In this study, sonodynamically induced selective antitumour effect of 5-ALA for deep-seated lesions was evaluated. MATERIALS AND METHODS: First, normal rat brains were sonicated via a transducer placed on the dural surface to confirm safe acoustic conditions for normal rat brains. One week after inoculation of brains with C6 rat glioma cells, brains with/without administration of 5-ALA (100 mg/kg body weight) were sonicated. RESULTS: Sonodynamic therapy (SDT) with 5-ALA and focused ultrasound (10 W/cm(2), 1.04 MHz, 5 min) achieved selective antitumour effect against deep-seated experimental glioma. Mean tumour sizes in the largest coronal section in sham-operated rats and rats receiving ultrasound with/without 5-ALA were 29.94±10.39, 18.32±5.69 and 30.81±9.65 mm(2), respectively. Tumour size was significantly smaller in the SDT group than in other groups (p<0.05). CONCLUSION: This experimental rat model showed that SDT appears to be useful in the treatment of deep-seated malignant glioma.

Radiation-induced astrocytoma with rapid malignant transformation: case report.

A 23-year-old man was admitted with a rare case of radiation-induced astrocytoma manifesting as 3-month history of unstable gait. He had received 50 Gy of irradiation therapy for a germ cell tumor in the right basal ganglia 13 years earlier. Magnetic resonance (MR) imaging on admission showed a non-enhanced mass lesion in the right cerebellar hemisphere with expansion to the vermis. The histological diagnosis of the stereotaxic biopsy specimen was grade II astrocytoma. Two months later, he developed drowsiness, and MR imaging demonstrated that the tumor had enlarged and was enhanced after gadolinium injection. The clinical diagnosis was high-grade glioma resulting from malignant transformation. The tumor had compressed the mesencephalic aqueduct, leading to obstructive hydrocephalus. Endoscopic third ventriculostomy was performed to improve the cerebrospinal fluid circulation. He underwent chemotherapy with temozolomide postoperatively, but died 8 months after the initial diagnosis of astrocytoma. The clinical course of radiation-induced astrocytoma is not benign. The potential for malignant transformation necessitates careful postoperative follow up for patients with this tumor.

Subependymoma with prominent rosenthal fiber formation: case report.

A 62-year-old woman presented with a rare case of subependymoma associated with prominent Rosenthal fibers located in the left lateral ventricle manifesting as right hemiparesis and mild motor aphasia. The tumor was well demarcated and consisted of clusters of round nuclei embedded in an abundant gliofibrillary matrix with some microcysts and prominent Rosenthal fibers. Immunohistochemically, the tumor stained positively for glial fibrillary acidic protein and negatively for synaptophysin. This case of subependymoma containing Rosenthal fiber formation is very unusual.

Endoscopic management of a traumatic meningo-encephalocele through a planum sphenoidale defect: case report.

A 37-year-old man involved in a motor vehicle accident was admitted to our hospital with disturbed consciousness. Computed tomography (CT) showed an acute, right-sided epidural hematoma and pneumocephalus. Emergency evacuation of the acute epidural hematoma was carried out, and his condition gradually recovered. However, cerebrospinal fluid (CSF) rhinorrhea continued despite conservative treatment. Three-dimensional reconstructed CT revealed a bone defect, which was approximately 20 mm in size, in the planum sphenoidale and tuberculum sellae, and magnetic resonance imaging demonstrated a traumatic meningo-encephalocele through the defect, with CSF collection in the sphenoid sinus. Endoscopic endonasal transsphenoidal surgery was performed. The 9-mm diameter dural defect was clearly visualized in the sphenoid sinus and was reconstructed with a combination of suturing and overlay with abdominal fascia, and absorbable polyglycolic acid felt. The CSF leakage from a traumatic meningo-encephalocele through the planum sphenoidale was successfully repaired by endoscopic endonasal surgery. Thorough preoperative evaluation of the feasibilities of the endoscopic and transcranial approaches should be based on the preoperative identification of the fistula, the bone defect, and vital structures.

Clinicopathological characteristics in patients presenting with acute onset of symptoms caused by Rathke's cleft cysts.

BACKGROUND: Symptomatic Rathke's cleft cyst is usually accompanied by a long history of headache, visual disturbance, and hypopituitarism; however, rare cases present with acute onset and the clinical features in such cases remain uncertain. We report herein the clinical features of Rathke's cleft cyst with acute onset and discuss the clinical significance. METHOD: In this study, we defined acute onset as the clinical course with clinical symptoms within a 7-day history. From among 35 cases of symptomatic Rathke's cleft cyst that were pathologically diagnosed at Fukuoka University Hospital between 1990 and 2009, five cases presented with acute onset. The symptoms, endocrinological findings, MR image findings, and pathological findings of these cases were analyzed retrospectively. FINDINGS: Mean age was 56.8 years. Initial symptoms included headache (n = 3), general malaise (n = 2), polyuria (n = 2), and fever (n = 1). MR imaging revealed an intrasellar cystic lesion with suprasellar extension in all cases and showed rim enhancement in three cases. All cases were treated by transsphenoidal surgery. Pathological findings included hemorrhage (n = 2), hypophysitis (n = 2), and abscess formation in the cyst (n = 1). Postoperatively, all symptoms, except for hypopituitarism, improved in all cases. CONCLUSIONS: Rathke's cleft cysts sometimes present with acute onset, and the presentation is consistent with the features of pituitary apoplexy caused by pituitary adenoma. Although pituitary apoplexy due to hemorrhage, inflammation, or infection due to an underlying Rathke's cleft cyst is difficult to diagnose pre-operatively, Rathke's cleft cyst should be included in the differential diagnosis, and early surgical treatment is needed, as for pituitary apoplexy caused by pituitary adenoma.

[A case of herpetic meningoencephalitis associated with massive intracerebral hemorrhage during acyclovir treatment: a rare complication].

We report a case of a 35-year-old female with herpetic meningoencephalitis confirmed by polymerase chain reaction and immunohistochemical study for herpes simplex virus-1 accompanied with a massive intracerebral hematoma as a complication. A hematoma localized at the medial temporal lobe and the medial frontal lobe occurred on the 11th day after initiation of acyclovir treatment. She subsequently required emergency surgery for temporal lobectomy, as well as hematoma and external decompression. Intracerebral hematoma with MR imaging showed gyral pattern along the cortex of the medial temporal lobe and the base of the medial frontal lobe. We speculate that the hemorrhage occurred by rupture of small vessels affected by vasculitis in addition to hypertension caused by increased intracranial pressure. We therefore emphasize the risk of intracerebral hemorrhage at an early stage or during acyclovir treatment, especially during one or two weeks after initiation of the treatment, and the necessity of careful observation during these periods.

Primary yolk sac tumor within the lateral ventricle.

A 13-year-old girl presented with an exceedingly rare case of primary yolk sac tumor located within the lateral ventricle, manifesting as headache, nausea, and diplopia. Magnetic resonance imaging revealed a 4-cm-diameter solid enhanced mass within the left inferior horn of the lateral ventricle. The tumor was removed subtotally via left middle temporal corticotomy. The histological and immunohistochemical diagnosis was pure yolk sac tumor. The serum alpha-fetoprotein (AFP) level was elevated at 1957.2 ng/ml and the serum beta-human chorionic gonadotropin level was 4 mIU/ml after surgery. The patient underwent radiotherapy (whole brain, 30 Gy; tumor bed, 21 Gy; whole spinal axis, 30 Gy) and chemotherapy (ifosfamide, cisplatin, etoposide). After three treatment cycles, the serum AFP level had decreased to 4.5 ng/ml. However, the tumor recurred with cerebrospinal fluid dissemination after nine cycles of chemotherapy. She died 18 months after surgery. The possibility of germ cell tumor should be considered in pediatric patients with brain tumors occurring outside the pineal or suprasellar region.

Effects of focused ultrasound sonodynamic treatment on the rat blood-brain barrier.

BACKGROUND: Ultrasound has recently been applied to the treatment as well as the diagnosis of various pathologies, and its antitumor effects in the treatment of human cancer and experimental models of cancer have been demonstrated. In addition, it is possible that certain photosensitizers will enhance the antitumor effects of ultrasound. However, very few studies have been reported on how the blood-brain barrier is affected by sonodynamic therapy. The purpose of this study was to evaluate disruption of the blood-brain barrier with focused ultrasound with a photosensitizer, for clinical application of sonodynamic therapy to brain tumors. MATERIALS AND METHODS: Rat brains were subjected to focused ultrasound irradiation via a transducer with or without prior intravenous injection of photosensitizer, and lesions were examined histologically by electron microscopy. RESULTS: Electron microscopically, swelling of astroglial processes, denatured cells, protoplasm of endothelial cells, and mitochondria were observed in the center and border of regions of ultrasonic irradiation. There were numerous pinocytotic vesicles in the cytoplasm of the endothelial cells. In addition, disruption of the cytoplasmic membrane of endothelial cells and astroglia was found in these regions. CONCLUSION: These findings suggest that sonodynamic therapy with a photosensitizer affects the blood-brain barrier, and that blood vessel permeability increases not only as a result of destruction of the blood-brain barrier but also by disruption of the cytoplasmic membrane of endothelial cells.

Mechanism of photofrin-enhanced ultrasound-induced human glioma cell death.

BACKGROUND: Low-intensity ultrasound showed tumor cell killing by a non-thermal effect in human leukemia cells. The aim of our study was to investigate the efficacy of low-intensity ultrasound on malignant astrocytic tumor cells with the photosensitizer, Photofrin, which is taken up by the cell surface receptor, low density lipoprotein receptor-related protein/alpha2-macroglobulin receptor (LRP/alpha2MR). MATERIALS AND METHODS: Cells were sonicated with continuous wave ultrasound with or without the presence of Photofrin (75 mg/ml) at an intensity of 0.3 W/cm(2) for a duration of 5, 15, or 30 s. RESULTS: Ultrasound alone induced instant cell killing immediately after sonication in both U251MG and U105MG malignant gliomas cells. In U251MG cells, which expressed LRP/alpha2MR, significant enhancement of cell killing was observed following Photofrin pretreatment, 52.7+/-17.5%, 13.0+/-4.6% and 3.9+/-0.9% for 5, 15, and 30 s respectively (p<0.05). This enhancement of cell killing was abolished by preincubation with receptor-associated protein (RAP) which binds specifically to LRP/alpha2MR. This enhancement by Photofrin was not achieved in U105MG which did not express LRP/alpha2MR. U251MG cells accumulated 2.43+/-0.25 Photofrin mg/mg protein, which significantly decreased with RAP pretreatment (1.38+/-0.22 Photofrin mg/mg protein) (p<0.05). U105MG cells accumulated 1.31+/-0.16 Photofrin mg/mg protein, which was significantly less than in U251MG cells. Photofrin uptake was not altered by RAP pretreatment in U105MG cells. U251MG cells exposed to ultrasound in the presence of Photofrin showed multiple surface pores and dimple-like craters. CONCLUSION: This is the first report to demonstrate the usefulness of low-intensity ultrasound for the cell killing of malignant glioma cells. Antitumor activity might be enhanced by combination with photosensitizer, which is transported by cell surface LRP/alpha2-MR to some degree.

Efficacy of temozolomide treatment in patients with high-grade glioma.

BACKGROUND: Numerous studies have reported the clinical efficacy of temozolomide (TMZ) treatment for high-grade glioma, but information on Japanese populations has been limited. This study assessed the safety and early outcomes of TMZ treatment, with or without combination therapy. PATIENTS AND METHODS: The subjects comprised ten patients with high-grade glioma [glioblastoma multiforme (GBM), n=3, gliosarcoma (GS), n=1, anaplastic oligodendroglioma (AO), n=3, anaplastic mixed oligoastrocytoma (AOA), n=1, and anaplastic ependymoma (AE), n=2]. All the patients were initially treated with conventional radiotherapy following surgical resection with or without adjuvant chemotherapy. As second- or third-line chemotherapy, patients received TMZ for recurrence or tumor progression. As combination therapy, the local administration of tumor necrosis factor-alpha and the addition of carboplatin and etoposide were included for three patients during the course of oral TMZ treatment. RESULTS: Partial response (PR) to TMZ therapy was achieved by four out of the ten patients (objective response rate, 40%), while three patients displayed stable disease (SD) and three showed disease progression (PD). One of the patients receiving combination therapy has continued to show shrinkage of the relapsed tumor. Despite prior radio- and chemotherapy, most patients experienced only grade 1-2 hematotoxicity that was well-controlled by conservative therapy. CONCLUSION: TMZ chemotherapy is effective for the treatment of high-grade glioma in some patients without serious toxicity. Assessing the true efficacy of TMZ will require a larger study with comparison of long-term outcomes between other agents or combined therapeutic modalities.

Management of pilomyxoid astrocytomas: our experience.

BACKGROUND: Pilomyxoid astrocytoma (PMA) shows a higher rate of recurrence and cerebrospinal fluid (CSF) dissemination than does pilocytic astrocytoma (PA). In this article, we discuss the treatment of PMA. MATERIALS AND METHODS: Between 1992 and 2007, the authors treated 5 patients. Two of these were male, three female. Their ages ranged from 3 months to 11 years. RESULTS: Three patients showed CSF dissemination on the initial radiographic examination. All patients received chemotherapy; the most commonly used combination drugs were cisplatin (CDDP)/carboplatin (CBDCA) and etoposide. When these drugs were unsuccessful, they were changed or other drugs added to the combination. After chemotherapy, four patients showed remarkable tumor regression. Nevertheless, one patient died 22 months after initial diagnosis, due to tumor progression. CONCLUSION: While our series was limited to a small number of patients, we have a positive impression of the value of chemotherapy. Even if initial chemotherapy is ineffective, we recommend continued CDDP/CBDCA-based chemotherapy with new drug combinations.

Sonodynamic therapy consisting of focused ultrasound and a photosensitizer causes a selective antitumor effect in a rat intracranial glioma model.

UNLABELLED: In this study we sought to determine the optimal focused ultrasound acoustic conditions with photosensitizers for the ablation of experimental intracranial glioma in rats. MATERIALS AND METHODS: Normal rat brains were sonicated via a transducer placed on the dural surface with or without a prior intravenous injection of the photosensitizer Rose Bengal (50 mg/kg of body weight). The ultrasound intensity was varied to 25, 110 or 150 W/cm(2), and the duration of irradiation was 10 s, or 1, 3, or 5 min. In experimental intracranial gliomas, one week after inoculation of C6 rat glioma cells in the rat brain, the rat brain was sonicated through a 10 mm-diameter craniotomy. RESULTS: A selective antitumor effect against cerebral glioma while sparing normal brain tissues was achieved by sonodynamic focused therapy consisting of focused ultrasound at 25 W/cm(2) at 1 MHz for 5 min and Rose Bengal (50 mg/kg of body weight). The areas of tumors in sham-operated rats and in rats that received sonodynamic therapy without and with Rose Bengal at an intensity of 25 W/cm(2) for 5 min were 19.53+/-3.89, 10.64+/-2.21 and 3.01+/-1.74 mm(2), respectively. The tumor area was significantly smaller in the ultrasound therapy groups than in control non-treated animals (p=0.002). There was no significant temperature change in tumor tissues during sonication with 25 W/cm(2) at 1 MHz. CONCLUSION: This is the first report to demonstrate the usefulness of sonodynamic therapy consisting of focused ultrasound and photosensitizer for the treatment of experimental malignant glioma.

A case of a heavily pigmented orbital melanocytoma.

We present an extremely rare case of an orbital melanocytoma that occurred in a 51-year-old man. The patient suffered from diplopia and mild exophthalmos of the right eye for 2 months. Brain magnetic resonance imaging showed a well-demarcated round mass 3.5 cm in diameter in the right orbit. We performed total resection of this tumor. Histological findings revealed a proliferation of large polygonal cells with fine pigment granules in the cytoplasm and prominent nucleoli. Although these tumor cells revealed immunohistochemical reactivity in HMB-1, there was no S-100 or Melan A antibody reactivity. Also, there were no malignant findings of nuclear polymorphism, mitoses, or necrosis. The brown pigments were confirmed to be melanin by bleaching and the Fontana-Masson silver stain method. The MIB-1 labeling index was less than 1%. This tumor also consisted of 50% melanophages, which revealed immunohistochemical reactivity in CD68, CD163, and in (1-AT antibodies. These histological findings led us to diagnose an orbital melanocytoma with partial tumor regression.

Survival and late effects on development of patients with infantile brain tumor.

BACKGROUND: Most infants with brain tumor may have a poor prognosis. The aim of the present study was to retrospectively analyze the survival and outcome with regard to mental and physical development in 11 subjects with brain tumor; these tumors were diagnosed when the patients were under 1 year of age. METHODS: The histological diagnoses of these tumors were astrocytoma, n = 3; pineocytoma, n = 2; teratoma, n = 1; ependymoma, n = 1; atypical teratoid/rhabdoid tumor, n = 1; glioblastoma, n = 1; medulloblastoma, n = 1; and choroid plexus papilloma, n = 1. Surgical resection was performed in eight patients, and adjuvant chemotherapy was administered to all except one patient with choroid plexus papilloma. Radiotherapy was additionally performed for four of the 10 chemotherapy patients. RESULTS: Six patients survived. Among the surviving patients, five were under no treatment for 50-167 months after the diagnosis (median duration, 89 months), while one received chemotherapy for 20 months. Five patients exhibited mental retardation, and one patient experienced normal development after surgical removal of his choroid plexus papilloma. Diencephalic syndrome developed in one patient with pilomyxoid astrocytoma that necessitated hormone replacement therapy, and bodyweight over +2 SD was observed in two patients. The remaining five patients died 11-111 months after diagnosis (median duration, 24 months). CONCLUSION: The prognosis of infantile brain tumor with regard to mortality and developmental outcome remains poor. Furthermore, survivors require comprehensive medical and social support for an extended period.

Ruptured tectal arteriovenous malformation demonstrated angiographically after removal of an unruptured occipital lobe arteriovenous malformation.

We report a case of ruptured tectal arteriovenous malformation (AVM) that was demonstrated angiographically only after removal of an unruptured occipital AVM. A 57-year-old man presented with sudden onset of diplopia and tinnitus. Computed tomography revealed a small hemorrhage in the right tectum mesencephali with intraventricular hemorrhage. Magnetic resonance imaging and angiography disclosed AVM in the right occipital lobe which was separate from the hemorrhagic lesion. Angiography demonstrated that the right occipital AVM was fed by the parieto-occipital artery and drained into the superior sagittal sinus and vein of Galen. However, no abnormal vascular lesion was detected near the tectum mesencephali. As venous hypertension was considered the reason for hemorrhage, the occipital AVM was completely resected. Postoperative angiography demonstrated disappearance of the occipital AVM, but it also disclosed a small tectal AVM fed by branches from the superior cerebellar artery, which had not been detected on preoperative angiography. This was considered the true cause of hemorrhage, and gamma knife surgery was accordingly performed. Even if an AVM is demonstrated, if the lesion does not correspond to the hemorrhage we recommend serial angiographical evaluation so that a small AVM is not missed.